Materiały źródłowe

• #1 Hemochromatosis (Iron Overload): Causes, Symptoms, Treatment, Diet & More

  https://my.clevelandclinic.org/health/diseases/14971-hemochromatosis-iron-overload

  The outlook for hemochromatosis depends on the timing of diagnosis and treatment. If not caught and addressed early, severe hemochromatosis can cause serious problems. These complications can include organ damage and possible death. […] But hemochromatosis is also a manageable disease. With early detection and treatment, you can survive and live a normal, healthy life. Sometimes organ damage can even be reversed. […] Your healthcare provider will determine how long you need treatment. […] If you are diagnosed with hemochromatosis, you’ll need to have some blood withdrawn once a week to start. You may then be able to switch to every few months, though you’ll still need ongoing treatment. […] Hemochromatosis is a common disease that makes the body store too much iron. Although it can cause serious problems, its very treatable, especially when identified early. Talk to your healthcare provider if you have symptoms of hemochromatosis or a family history of it. Genetic testing can identify the disease early so you can live a long, healthy life.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics

  HEMOCHROMATOSIS TREATMENT […] Treatment typically involves regular phlebotomy (removal of blood) since red blood cells contain a large amount of the body’s iron. […] Treatment of hemochromatosis requires removal of excessive iron from the body, usually by periodically removing blood (referred to as „therapeutic phlebotomy”). […] Therapeutic phlebotomy entails periodic removal of blood. […] Phlebotomy removes iron by removing red blood cells, which contain significant amounts of iron. […] The decision to begin phlebotomy in a person with hemochromatosis is usually based on a person’s age, sex, level of ferritin in the blood, and results of magnetic resonance imaging (MRI) or liver biopsy. […] Phlebotomy is appropriate and beneficial for most people with excess iron stores from hemochromatosis, including older adults and people who have no symptoms.

• #1 Treatment of Hemochromatosis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/hemochromatosis/treatment

  In most cases, doctors treat hemochromatosis with phlebotomy, or drawing about a pint of blood at a time, on a regular schedule. This is the most direct and safe way to lower body stores of iron. […] Treatment of hemochromatosis can improve symptoms and prevent complications. […] Phlebotomy removes extra iron from your blood. Phlebotomy is simple, inexpensive, and safe. […] After phlebotomy has removed extra iron and blood levels of iron and ferritin return to normal, doctors will reduce phlebotomies to once every 1 to 3 months and eventually to 2 to 3 times a year. […] To treat secondary hemochromatosis in these people, doctors prescribe medicines, called chelating agents, that bind to iron and allow it to pass from the body in urine. […] Doctors treat neonatal hemochromatosis in newborns with exchange transfusions removing blood and replacing it with donor blood and IV immunoglobulin a solution of antibodies from healthy people.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics/print

  Early identification and treatment of hemochromatosis can prevent complications and ensure a normal life expectancy. Treatment typically involves regular phlebotomy (removal of blood) since red blood cells contain a large amount of the body’s iron. […] Treatment of hemochromatosis requires removal of excessive iron from the body, usually by periodically removing blood (referred to as „therapeutic phlebotomy”). […] Therapeutic phlebotomy entails periodic removal of blood. The process is almost identical to donating blood (and involves removing about the same amount). […] Phlebotomy removes iron by removing red blood cells, which contain significant amounts of iron. The body uses up stored iron to make up for the loss of red blood cells, thereby gradually depleting iron stores. Over time, phlebotomy reduces iron stores back to normal levels.

• #1 Haemochromatosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/haemochromatosis/

  Treatment for haemochromatosis is simply aimed at removing iron from your body. As the body has no natural method for getting rid of the extra iron, this is done by regular bleeding known as phlebotomy. […] During phlebotomy a unit of blood, usually 450 millitres (ml), is removed. This amount will contain 220mg of iron. Bleeding in this way will activate the remaining stored iron to make new red blood cells. […] You will be required to have phlebotomy up to once a week, depending on the degree of your iron overload. This may continue for up to two years. Over this period doctors will monitor your serum ferritin levels until they fall to a safe level (generally 20 mcg per litre). Removing blood does not stop the iron building up. […] After your course of treatment you will need to have further phlebotomies two to four times a year for the rest of your life. Doctors will continue to monitor transferrin saturation and serum ferritin levels (ideally maintained at 50% and 50 mcg per litre respectively) to assess when phlebotomy may be required. This is known as maintenance therapy.

• #1

  https://www.nhs.uk/conditions/haemochromatosis/treatment/

  There’s currently no cure for haemochromatosis, but there are treatments that can reduce the amount of iron in your body. […] The most commonly used treatment for haemochromatosis is a procedure to remove some of your blood, known as a venesection or phlebotomy. […] There are 2 main stages to treatment: induction blood is removed on a frequent basis (usually weekly) until your iron levels are normal; this can sometimes take up to a year or more […] maintenance blood is removed less often (usually 2 to 4 times a year) to keep your iron levels under control; this is usually needed for the rest of your life. […] A treatment called chelation therapy may be used in a small number of cases where regular phlebotomies are not possible because it’s difficult to remove blood regularly for example, if you have very thin or fragile veins. […] Commonly used medicines are deferasirox and desferrioxamine. […] Deferasirox is unlicensed for the treatment of haemochromatosis, which means it has not undergone extensive clinical trials for this use. But your doctor may recommend it if they feel the possible benefits outweigh any risks.

• #1 Hemochromatosis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/hemochromatosis

  The treatment of HH is simple and relatively safe. Therapeutic phlebotomy will effectively mobilize and remove iron stores and, when adhered to on a regular basis, will maintain them at normal levels. Patients should be encouraged to adhere to a regimen of phlebotomy of one unit of blood once or twice weekly as tolerated initially. This will remove approximately 250 mg of iron for each unit of phlebotomy, depending on the starting hematocrit value. In situations in which total body iron stores exceed 20 to 30 g, this regimen of phlebotomy may take up to 2 to 3 years to complete. The aim is to reduce iron stores to a level just short of iron deficiency. The hematocrit value should be monitored before each phlebotomy and should be postponed if it falls by more than 20% of its starting value. It is reasonable to check the serum ferritin level after every 10 to 12 phlebotomies. The serum ferritin level may be expected to fall progressively with iron mobilization, and it can be confidently assumed that effective mobilization of the iron stores will be completed when the serum ferritin level falls below 50 ng/mL.

• #1 Hemochromatosis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/hemochromatosis

  Subsequently, a maintenance schedule may be initiated, and it can be expected that a one-unit phlebotomy may be necessary every 2 to 3 months. The aim of maintenance therapy is to keep the serum ferritin level between 25 and 50 ng/mL, thereby avoiding overt iron deficiency. Currently, phlebotomy is a therapeutic procedure, with a coding recognized by the Centers for Medicare and Medicaid Services (formerly the Health Care Finance Administration) and third-party insurers. Although there is continued elevation of the TS level until late in the course of therapy, it is important to avoid pharmacologic doses of vitamin C, which may result in accelerated mobilization of iron. This might saturate the circulating transferrin and lead to potentially toxic complications, such as cardiac dysrhythmias and cardiomyopathy. Deferoxamine (Desferal) is an iron-chelating agent reserved for those with secondary iron overload caused by dyserythropoietic anemia. Finally, there are many specialists who prescribe erythropoietin, which is given systemically to promote red cell production in those who are unable to mount a bone marrow response to phlebotomy.

• #1 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  The AASLD guidelines state hereditary hemochromatosis patients who have evidence of iron overload are strongly encouraged to receive phlebotomy regularly until iron stores are depleted. […] In patients with hemochromatosis and heart disease, anemia, or poor venous access, treatment with iron chelation agents is recommended. […] Deferasirox (Exjade) is the oral iron chelator that should be taken once daily as an adjunct to phlebotomies or instead of phlebotomy in patients in whom these procedures are poorly tolerated. […] Dietary factors may influence the phenotypic expression of hemochromatosis. […] Promptly refer patients to a gastroenterologist and a liver transplant center in case of end-stage liver disease, especially if it is refractory to treatment. […] Regular monitoring of hematocrit, hemoglobin, and serum ferritin levels is necessary in patients undergoing phlebotomy.

• #1 Hereditary Hemochromatosis: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0900/p263.html

  Phlebotomy is the mainstay of treatment and can help improve heart function, reduce abnormal skin pigmentation, and lessen the risk of liver complications. […] Phlebotomy should be offered to patients with elevated serum ferritin levels (greater than 300 ng per mL in men and greater than 200 ng per mL in women) or a transferrin saturation of greater than 45% in both men and women. […] The American College of Gastroenterology recommends weekly removal of 500 mL of blood, increasing or decreasing the amount based on patient tolerance. […] Monthly ferritin levels should be tracked, with weekly blood removal continuing until a serum ferritin level of 50 to 150 ng per mL (50 to 150 mcg per L) is obtained. […] Once this goal is achieved, the patient should undergo lifelong phlebotomy three or four times per year, with ferritin monitoring every six months, to maintain levels around 50 ng per mL.

• #1 Hemochromatosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemochromatosis/diagnosis-treatment/drc-20351448

  Medical professionals can treat hemochromatosis safely and effectively by removing blood from the body on a regular basis. This is similar to donating blood. The process is known as phlebotomy. […] The goal of phlebotomy is to lower the iron levels. The amount of blood removed and how often it’s removed depend on age, overall health and the severity of iron overload. […] Treating hemochromatosis can help relieve symptoms of tiredness, stomach pain and skin darkening. It can help prevent serious complications such as liver disease, heart disease and diabetes. If you already have one of these conditions, phlebotomy may slow the progression of the disease. In some cases, it may even reverse it. […] Phlebotomy can’t reverse cirrhosis or joint pain, but it can slow the progression. […] Phlebotomy may not be an option for someone who has certain conditions, such as anemia or heart complications. Instead, a healthcare professional may recommend a medicine to remove excess iron. The medicine can be injected into the body, or it can be taken as a pill. The medicine binds excess iron, allowing the body to expel iron through urine or stool in a process that’s called chelation (KEE-lay-shun). Chelation is not commonly used in hemochromatosis.

• #1 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics

  After excess iron has been removed, maintenance phlebotomy is used to prevent iron from reaccumulating. […] Phlebotomy can effectively remove iron and resolve some, but not all, complications of hemochromatosis. […] Chelation therapy refers to treatment with a drug that removes iron from the body. […] Chelation is sometimes used in people who have iron overload but cannot undergo phlebotomy because they also have heart failure or anemia. […] Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #1 HFE hemochromatosis: an overview about therapeutic recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8885398/

  A phlebotomy schedule on the order of 400 – 500mL, considering body weight, weekly or every two weeks has been proposed. […] One phlebotomy every 1 to 4 months, depending on the patient’s iron status. […] Patients who have had iron overload should never stop checking his or her iron parameters and should be followed lifelong on an out-patient basis. […] Iron chelation therapy is not indicated for classical hemochromatosis. […] However, in rare cases, iron chelators are an adjuvant treatment (or alternative), such as in severe iron overload without efficacy with phlebotomies, poor vein conditions and severe non-HFE hemochromatosis (mainly juvenile form). […] Two current studies showed the importance of the adequate hemochromatosis treatment and of the early intervention to prevent morbidity caused by the hemochromatosis related to the HFE p.Cys282Tyr homozygosity.

• #1 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemochromatosis-Treatments.aspx

  Some patients are not fit for phlebotomy, as for instance, those with significant anemia or heart disease. In such cases iron removal from the body is facilitated by the use of certain compounds which bind iron in soluble form and the excess can be removed through urine or feces. The approved iron chelators are used in pill form or as injections. The injections are used in subcutaneous form and administered over 812 hours using a pump, which runs at night. Oral chelators may be taken by dissolving them in water or juice, once daily. Some newer formulations allow them to be swallowed as a pill on an empty stomach. Oral chelators are well tolerated however, should not be used in people with high-risk myelodysplastic syndrome. […] Iron chelators reduce body iron overload very effectively. However, an early side effect may include renal and liver injury and most side effects subside once the body accustoms itself to the drug. Yearly monitoring and retests for the baseline measurement of renal and liver function, visual and auditory testing is recommended to monitor such side effects.

• #1 Hemochromatosis (Iron Overload): Causes, Symptoms, Treatment, Diet & More

  https://my.clevelandclinic.org/health/diseases/14971-hemochromatosis-iron-overload

  Diet changes and other treatments can help ease the symptoms of hemochromatosis. They can also help prevent or delay further damage to your organs: […] Your healthcare provider will probably ask you to avoid supplements with iron. You may also need to stay away from foods with too much iron and limit vitamin C. Limit your alcohol consumption, too, because its not good for your liver. […] This medication removes extra iron from your body. Its taken by mouth at home or injected into the blood by a healthcare provider. […] This procedure uses a needle and tube to remove blood, and the iron it contains, from your body. Treatment needs to be repeated frequently, so you’ll have regular blood tests to measure iron levels. […] If another condition caused hemochromatosis, you may need treatment for that, too. And healthcare providers may recommend treating any problems caused by hemochromatosis.

• #1 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  Studies clearly show that both survival rates and quality of life are significantly improved with phlebotomy therapy, even for patients who have already sustained organ damage. […] In the future, patients with hemochromatosis may have other options for the type of treatment that is best for them, but for now, phlebotomy is the gold standard. […] However, it is important to modify lifestyle and diet in order to not take in more-than-normal amounts of iron through the diet such as in certain vitamin pills or fortified foods. […] In the active de-ironing phase, reducing iron intake is almost insignificant compared to the 225 mg of iron that are being removed with each phlebotomy. In the maintenance phase, restricting iron intake may increase the time between phlebotomies but at no time will reducing iron in the diet replace phlebotomies as effective therapy.

• #1 About Hereditary Hemochromatosis | Hereditary Hemochromatosis | CDC

  https://www.cdc.gov/hereditary-hemochromatosis/about/index.html

  If you have hemochromatosis, your healthcare provider may suggest ways to lower the amount of iron in your body. […] If you are diagnosed with hemochromatosis, regularly scheduled blood removal is the most effective way to lower the amount of iron in your body. […] Your healthcare provider may also recommend annual blood tests to check your iron levels. […] Iron chelation therapy, if you cannot have blood removed. This involves medicine taken either orally or injected to lower the amount of iron in your body. […] Dietary changes, such as avoiding multivitamins, vitamin C supplements, and iron supplements, which can increase iron throughout your body. […] No alcohol use (because alcohol increases the risk of liver damage). […] Steps to prevent infections, including not eating uncooked fish and shellfish and getting recommended vaccinations, including those against hepatitis A and B.

• #1 Hereditary Hemochromatosis: Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2021/0900/p263.html

  Phlebotomy may improve heart function, reduce abnormal skin pigmentation, and lessen the risk of liver complications. […] Liver transplantation may be considered in patients who develop end-stage liver disease or hepatocellular carcinoma. […] A small study demonstrated that liver transplantation can normalize hepcidin secretion and prevent recurrence of hepatic iron overload.

• #1 Haemochromatosis and haemochromatotic arthritis — Arthritis Australia

  https://arthritisaustralia.com.au/types-of-arthritis/haemochromatosis-and-haemochromatotic-arthritis/

  The goal of treatment for haemochromatosis is to reduce iron levels to a safe level and prevent long term complications of iron overload. Treatment for haemochromatosis usually involves removal of blood via a needle into the arm, similar to blood donation. Up to 500mL of blood is removed at regular intervals until iron levels are at a safe level. […] Although this type of treatment is usually very effective for iron levels, it does not appear to reduce the symptoms of arthritis or reverse the damage to the joints. […] Treatments for arthritis vary depending on which joints are affected and can include: pain relief, using medicines such as paracetamol; non-steroidal anti-inflammatory drugs (NSAIDs), including topical creams and ointments; an exercise program tailored to your needs and ability; a weight loss program, if you are overweight; joint replacement surgery, if your symptoms are no longer controlled with other therapies.

• #1 Researchers find potential cure for deadly iron-overload disease | News Center

  https://med.stanford.edu/news/all-news/2020/09/researchers-find-potential-cure-for-deadly-iron-overload-disease.html

  „Hemochromatosis can be treated by iron chelation therapy, but for many patients this is not an option due to concurrent kidney problems,” Rhee said. […] „Chelation therapy is, unfortunately, toxic to the kidneys,” Rhee said. „Angelina’s liver disease had also caused kidney failure, so the chelation therapy wasn’t a safe option. That also is not uncommon.” […] In a recently published study in Cell Reports, Rhee and her colleagues at the Stanford School of Medicine determined that a drug called ebselen may be able to prevent heart failure in patients with iron-overload disease. […] Finally, the researchers exposed these heart cells to a series of drugs, looking for an agent to reverse the iron-overload process. Ebselen proved to be the best candidate for blocking the channels for iron entry, potentially protecting against heart failure.

• #1 Advancements in Hemochromatosis: A Comprehensive Overview of Clinical Trials

  https://www.lindushealth.com/blog/advancements-in-hemochromatosis-a-comprehensive-overview-of-clinical-trials

  While phlebotomy remains an essential treatment option, advancements in medical research have introduced alternative therapies, including iron chelation therapy and hepcidin mimetics. Iron chelation therapy involves the use of medications to bind and remove excess iron from the body, while hepcidin mimetics target the regulation of iron absorption. […] Iron chelation therapy, for example, has shown promising results in reducing iron levels in patients who cannot tolerate or do not respond well to phlebotomy. […] Moreover, hepcidin mimetics represent a groundbreaking approach to hemochromatosis treatment. By targeting the regulation of iron absorption, these medications offer a more targeted and precise method of managing iron overload. […] Novel drug therapies are currently being investigated as potential treatments for hemochromatosis. These include iron chelators with improved specificity and reduced side effects.

• #1 Iron overload – Wikipedia

  https://en.wikipedia.org/wiki/Iron_overload

  Medications are used for those unable to tolerate routine blood draws, there are chelating agents available for use. The drug deferoxamine binds with iron in the bloodstream and enhances its elimination in urine and faeces. Typical treatment for chronic iron overload requires subcutaneous injection over a period of 8-12 hours daily. Two newer iron-chelating drugs that are licensed for use in patients receiving regular blood transfusions to treat thalassaemia (and, thus, who develop iron overload as a result) are deferasirox and deferiprone. […] A minimally invasive approach to hereditary hemochromatosis treatment is the maintenance therapy with polymeric chelators. These polymers or particles have a negligible or null systemic biological availability and they are designed to form stable complexes with Fe2+ and Fe3+ in the GIT and thus limiting their uptake and long-term accumulation. Although this method has only a limited efficacy, unlike small-molecular chelators, the approach has virtually no side effects in sub-chronic studies. Interestingly, the simultaneous chelation of Fe2+ and Fe3+ increases the treatment efficacy.

• #1 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  Despite advances in the molecular understanding of hemochromatosis and the impact of C282Y on diagnosis, treatment remains simple, inexpensive, and safe. […] The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage. This is achieved via chelation therapy or venesection, depending on the underlying cause. […] A Cochrane database review of interventions for hereditary hemochromatosis found that phlebotomy remained the treatment of choice in those with hereditary hemochromatosis who required blood letting, but no data from randomized trials provided evidence of benefit from any form of blood letting in these patients. […] Once diagnosed, hemochromatosis is treated by phlebotomy to rid the body of excess iron and to maintain normal iron stores. Phlebotomy remains the sole recommended treatment for hereditary hemochromatosis and should be undertaken in a case-specific manner.

• #1 Haemochromatosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemochromatosis

  If a person is diagnosed before significant symptoms arise, they can prevent organ damage and disease symptoms by maintaining iron in the normal range. However, a person diagnosed with the condition should notify all blood relatives so they can be tested for the HFE genes and treated if necessary. Prompt treatment can reverse some organ damage and symptoms, and prevent further damage.

• #2 Hemochromatosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemochromatosis/symptoms-causes/syc-20351443

  Treatment includes regularly removing blood from the body. Because much of the body’s iron is contained in red blood cells, this treatment lowers iron levels. […] Untreated, hemochromatosis can lead to several complications. These complications especially affect the joints and organs where excess iron tends to be stored, such as the liver, pancreas and heart.

• #2 Hereditary Hemochromatosis – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/iron-overload/hereditary-hemochromatosis

  Treatment is usually with serial phlebotomies. […] Treatment is indicated for patients with clinical manifestations, elevated serum ferritin levels (particularly levels 1000 ng/mL [ 1000 mcg/L]), or elevated transferrin saturation 45%. […] Phlebotomy is the simplest and most effective method to remove excess iron. […] Phlebotomy (either weekly or every other week) may be needed for many months (eg, if 250 mg of iron are removed per week, 40 weeks will be required to remove 10 g of iron). […] When iron levels are normal, phlebotomy can be done intermittently to maintain the ferritin level between 50 and 100 ng/mL. […] Diabetes mellitus, cardiomyopathy, erectile dysfunction, and other secondary manifestations are treated as indicated. […] Patients should follow a balanced diet; it is not necessary to restrict consumption of iron-containing foods (eg, red meat, liver).

• #2 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  Despite advances in the molecular understanding of hemochromatosis and the impact of C282Y on diagnosis, treatment remains simple, inexpensive, and safe. […] The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage. This is achieved via chelation therapy or venesection, depending on the underlying cause. […] A Cochrane database review of interventions for hereditary hemochromatosis found that phlebotomy remained the treatment of choice in those with hereditary hemochromatosis who required blood letting, but no data from randomized trials provided evidence of benefit from any form of blood letting in these patients. […] Once diagnosed, hemochromatosis is treated by phlebotomy to rid the body of excess iron and to maintain normal iron stores. Phlebotomy remains the sole recommended treatment for hereditary hemochromatosis and should be undertaken in a case-specific manner.

• #2 Treatment and Maintenance – Canadian Hemochromatosis Society

  https://www.toomuchiron.ca/hemochromatosis/treatment-and-maintenance/

  Treatment for hemochromatosis involves management of complications, screening for liver cancer, avoidance of supplemental iron and appropriate vaccinations for hepatitis A and B; however, an aggressive de-ironing protocol is most important. Excess iron is removed by a procedure known as phlebotomy, which is the drawing off of a unit of blood, using the same technique as a blood donation, but with a much higher frequency. […] This treatment is effective because phlebotomies remove red blood cells that contain iron. Each unit of blood contains approximately 225 mg of iron in hemoglobin, the main component of red blood cells. In the process of making new red blood cells, a signal is sent for the stored iron in the tissues and organs to be pulled out and transported to the bone marrow where red blood cells are produced. This repeated procedure gradually depletes the stores of excess iron and eventually the iron levels fall back to normal.

• #2 HFE hemochromatosis: an overview about therapeutic recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8885398/

  Hemochromatosis is currently characterized by the iron overload caused by hepcidin deficiency. […] In 2018, a Taskforce of the Hemochromatosis International (HI) published a practical set of recommendations on the therapeutic aspects of HFE hemochromatosis for the p.Cys282Tyr (C282Y/C282Y) homozygous genotype, based on published scientific studies and guidelines. […] The main aims of this study are to show updates on hemochromatosis and to report some aspects of the published therapeutic recommendations. […] Phlebotomy (venesection therapy) is the standard treatment for patients with hemochromatosis and it is very effective to prevent hemochromatosis damages, safe and has a low cost. […] Therapeutic recommendations from the HI Taskforce divided the treatment into two phases: initial phase (or induction) and maintenance phase.

• #2 HFE hemochromatosis: an overview about therapeutic recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8885398/

  A phlebotomy schedule on the order of 400 – 500mL, considering body weight, weekly or every two weeks has been proposed. […] One phlebotomy every 1 to 4 months, depending on the patient’s iron status. […] Patients who have had iron overload should never stop checking his or her iron parameters and should be followed lifelong on an out-patient basis. […] Iron chelation therapy is not indicated for classical hemochromatosis. […] However, in rare cases, iron chelators are an adjuvant treatment (or alternative), such as in severe iron overload without efficacy with phlebotomies, poor vein conditions and severe non-HFE hemochromatosis (mainly juvenile form). […] Two current studies showed the importance of the adequate hemochromatosis treatment and of the early intervention to prevent morbidity caused by the hemochromatosis related to the HFE p.Cys282Tyr homozygosity.

• #2 Hemochromatosis and why blood loss can be a form of therapy

  https://www.blood.ca/en/research/our-research-stories/research-education-discovery/hemochromatosis-and-why-blood-loss-can

  For those diagnosed with hemochromatosis, one way to reduce the excess iron is to limit eating foods or taking vitamin supplements that contain iron. But, the most effective treatment is to remove blood from the body. This type of therapy is called phlebotomy. […] Each patient is diagnosed with different levels of iron in their body, and so people may need regular phlebotomy treatments for different periods of time. But overall, phlebotomy, a treatment that makes use of the bodys natural blood production process, is quite effective. […] The gradual process of phlebotomy can effectively extract iron from the body of people with hemochromatosis, preventing further damage to vital organs. Some evidence even suggests that phlebotomy can reverse early tissue damage (fibrosis) in the liver. […] Phlebotomy provides therapeutic benefit to people with hemochromatosis, but also has the potential to benefit other people who are in need of a blood transfusion as well. […] Once a persons ferritin level is in a reasonable range, the frequency of phlebotomies can decrease. This is called maintenance therapy, which may involve phlebotomies every 3 to 4 months.

• #2 Hemochromatosis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/hemochromatosis

  The treatment of HH is simple and relatively safe. Therapeutic phlebotomy will effectively mobilize and remove iron stores and, when adhered to on a regular basis, will maintain them at normal levels. Patients should be encouraged to adhere to a regimen of phlebotomy of one unit of blood once or twice weekly as tolerated initially. This will remove approximately 250 mg of iron for each unit of phlebotomy, depending on the starting hematocrit value. In situations in which total body iron stores exceed 20 to 30 g, this regimen of phlebotomy may take up to 2 to 3 years to complete. The aim is to reduce iron stores to a level just short of iron deficiency. The hematocrit value should be monitored before each phlebotomy and should be postponed if it falls by more than 20% of its starting value. It is reasonable to check the serum ferritin level after every 10 to 12 phlebotomies. The serum ferritin level may be expected to fall progressively with iron mobilization, and it can be confidently assumed that effective mobilization of the iron stores will be completed when the serum ferritin level falls below 50 ng/mL.

• #2 Sign up for our monthly newsletter

  https://www.haemochromatosis.org.uk/genetic-haemochromatosis-care-pathway

  With treatment, many people live full and active lives with genetic haemochromatosis. […] For most people, treatment for genetic haemochromatosis is simple and effective; its a form of regular blood donation known as venesection. […] Initially, your doctor will ask you to attend hospital or a clinic weekly or fortnightly to have up to 1 unit (450 ml) of blood drawn. […] Venesection isn’t always an option for people (for example, if venous access is consistently troublesome); for a small number, alternative therapies including chelation therapy may be used instead (or interchangeably). […] Following a period of regular venesection, you will reach maintenance once your serum ferritin (SF) is below 100 g/l and transferrin saturation (TSAT) is below 50%. […] You will need to continue to have venesection periodically to maintain your iron levels at normal levels, ideally where serum ferritin (SF) is below 50 g/l and transferrin saturation (TSAT) is below 50%. […] Once in maintenance, many people can go on to be regular blood donors, helping others.

• #2 Treatment of Hemochromatosis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/hemochromatosis/treatment

  Phlebotomy can prevent the complications of hemochromatosis. […] Early treatment with phlebotomy can prevent complications of iron overload caused by these gene mutations. […] However, doctors can check iron levels and start treatment with chelating agents early, before iron overload causes damage to the liver, joints, and other organs.

• #2 Hemochromatosis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/hemochromatosis

  Phlebotomy can’t reverse cirrhosis or joint pain, but it can slow the progression. […] Phlebotomy may not be an option for someone who has certain conditions, such as anemia or heart complications. Instead, a healthcare professional may recommend a medicine to remove excess iron. The medicine can be injected into the body, or it can be taken as a pill. The medicine binds excess iron, allowing the body to expel iron through urine or stool in a process that’s called chelation (KEE-lay-shun). Chelation is not commonly used in hemochromatosis.

• #2 Hemochromatosis Treatment & Management: Approach Considerations, Surgical Intervention, Phlebotomy

  https://emedicine.medscape.com/article/177216-treatment

  The AASLD guidelines state hereditary hemochromatosis patients who have evidence of iron overload are strongly encouraged to receive phlebotomy regularly until iron stores are depleted. […] In patients with hemochromatosis and heart disease, anemia, or poor venous access, treatment with iron chelation agents is recommended. […] Deferasirox (Exjade) is the oral iron chelator that should be taken once daily as an adjunct to phlebotomies or instead of phlebotomy in patients in whom these procedures are poorly tolerated. […] Dietary factors may influence the phenotypic expression of hemochromatosis. […] Promptly refer patients to a gastroenterologist and a liver transplant center in case of end-stage liver disease, especially if it is refractory to treatment. […] Regular monitoring of hematocrit, hemoglobin, and serum ferritin levels is necessary in patients undergoing phlebotomy.

• #2 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics

  After excess iron has been removed, maintenance phlebotomy is used to prevent iron from reaccumulating. […] Phlebotomy can effectively remove iron and resolve some, but not all, complications of hemochromatosis. […] Chelation therapy refers to treatment with a drug that removes iron from the body. […] Chelation is sometimes used in people who have iron overload but cannot undergo phlebotomy because they also have heart failure or anemia. […] Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #2 Hemochromatosis: Signs, Causes and Treatment | Banner Health

  https://www.bannerhealth.com/services/hematology/hemochromatosis

  While chelation therapy is effective, it may have side effects like upset stomach or joint pain. Your provider will decide if this treatment is right for you. […] In addition to these treatments, your provider may recommend other lifestyle changes to help lower your iron intake: […] Regular blood tests can help monitor your iron levels and liver function, especially if you are at risk.

• #2 Haemochromatosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemochromatosis

  Treatment includes regularly removing blood until iron levels normalise. […] A person with haemochromatosis is treated with venesection. This is a procedure similar to blood donation, where around 500 mls of blood is removed until iron in the blood is reduced to normal levels. Depending on the severity of the condition, this may take around one and a half years of twice-weekly visits. Once iron levels are normal, venesection needs to be performed three or four times every year for life. If haemochromatosis is treated in its earliest stages before severe organ damage has occurred, there is no reduction in life expectancy other things being equal. […] A person with haemochromatosis can better manage their condition by making a few simple lifestyle changes, including: not taking iron supplements, not taking vitamin C supplements, as vitamin C increases iron absorption, reducing alcohol intake, as metabolising alcohol can stress an already compromised liver, reducing or limiting iron-rich foods such as offal.

• #2 Advancements in Hemochromatosis: A Comprehensive Overview of Clinical Trials

  https://www.lindushealth.com/blog/advancements-in-hemochromatosis-a-comprehensive-overview-of-clinical-trials

  Complementary approaches, such as dietary modifications and lifestyle changes, are also being explored to manage iron levels in individuals with hemochromatosis. […] Through rigorous studies and clinical trials, researchers have identified specific dietary modifications that can help individuals with hemochromatosis manage their iron levels more effectively. […] With continued advances in hemochromatosis treatment, patient care is expected to improve significantly. Early detection methods, coupled with targeted therapies, have the potential to prevent or delay the onset of complications associated with iron overload.

• #2 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics/print

  Phlebotomy can help prevent the potentially life-threatening complications of cirrhosis and liver cancer. […] Phlebotomy can resolve or markedly improve weakness, fatigue, lethargy, and darkening of the skin. […] Phlebotomy may resolve joint pain and heart disease. […] Chelation therapy refers to treatment with a drug that removes iron from the body. […] Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #2 Hemochromatosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/177216-overview

  Once diagnosed, hemochromatosis is treated by phlebotomy to rid the body of excess iron and to maintain normal iron stores. Phlebotomy remains the sole recommended treatment for hereditary hemochromatosis and should be undertaken in a case-specific manner. […] In patients with hemochromatosis and heart disease, anemia, or poor venous access, treatment with iron chelation agents is recommended and includes the following agents: Deferoxamine, Deferasirox, Deferiprone, Iron-binding dendrimers. […] Surgical procedures are used to treat two important complications of hemochromatosis: end-stage liver disease and severe arthropathy. Orthotopic liver transplantation is the only therapeutic option when end-stage liver disease progresses despite iron-reduction therapy. This intervention is also indicated in cases with the development of hepatocellular carcinoma. […] Surgical arthroplasty is considered if joint destruction becomes severe despite medical therapy.

• #2 Hemochromatosis – What You Need to Know

  https://www.drugs.com/cg/hemochromatosis.html

  Hemochromatosis cannot be cured. You may need the following to manage your symptoms: […] Phlebotomy is used to take small amounts of blood to help decrease the amount of extra iron in your body. You may need to have this done regularly, or when your iron levels get higher than your healthcare provider recommends. […] Medicines may be needed for other health conditions caused by hemochromatosis. Some examples are thyroid disease, high or low blood pressure, diabetes, impotence, and heart disease. […] The following list of medications are related to or used in the treatment of this condition. […] deferasirox

• #2 Advancements in Hemochromatosis: A Comprehensive Overview of Clinical Trials

  https://www.lindushealth.com/blog/advancements-in-hemochromatosis-a-comprehensive-overview-of-clinical-trials

  While phlebotomy remains an essential treatment option, advancements in medical research have introduced alternative therapies, including iron chelation therapy and hepcidin mimetics. Iron chelation therapy involves the use of medications to bind and remove excess iron from the body, while hepcidin mimetics target the regulation of iron absorption. […] Iron chelation therapy, for example, has shown promising results in reducing iron levels in patients who cannot tolerate or do not respond well to phlebotomy. […] Moreover, hepcidin mimetics represent a groundbreaking approach to hemochromatosis treatment. By targeting the regulation of iron absorption, these medications offer a more targeted and precise method of managing iron overload. […] Novel drug therapies are currently being investigated as potential treatments for hemochromatosis. These include iron chelators with improved specificity and reduced side effects.

• #2 Hemochromatosis Treatment- Phlebotomy, Natural Remedies | Hemochromatosis Help

  https://hemochromatosishelp.com/hemochromatosis-treatment/

  The goal of therapeutic phlebotomy is to remove blood in an attempt to clear stored iron from the tissues and organs. […] However, there are many reasons why someone may not be able to tolerate phlebotomy. […] Conventionally, if someone doesnt tolerate phlebotomy, doctors may choose to use a chelating medication. […] Chelating medicines work by binding to excess iron to help excrete it from the body. […] It is always important to follow your doctors advice. One of these medications may save your life, especially if you are unable to tolerate therapeutic phlebotomy. […] I absolutely agree that therapeutic phlebotomy is the cornerstone, front line, most important therapy in the treatment of hemochromatosis. By reducing excess iron, phlebotomy can reduce symptoms, halt or hopefully reverse the progress of the disease, and save lives.

• #2 Hemochromatosis | UCSF Department of Surgery

  https://hpbsurgery.ucsf.edu/condition/hemochromatosis

  Treating hemochromatosis before organs are damaged can prevent complications such as cirrhosis, heart problems, arthritis, and diabetes. Treatment cannot cure these conditions in patients who already have them at diagnosis. However, treatment will help most of these conditions improve. The treatments effectiveness depends on the degree of organ damage. For example, treating hemochromatosis can stop the progression of liver damage in its early stages and lead to a normal life expectancy. However, if a patient develops cirrhosis, his or her chance of developing liver cancer increases, even with phlebotomy treatment. Arthritis usually does not improve even after phlebotomy removes extra iron.

• #2 Living With Hemochromatosis | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-hemochromatosis/what-causes-hemochromatosis/living

  After the initial treatment period, you may need ongoing treatment two to six times a year. This will help prevent the iron from building up again. […] If you have hemochromatosis, getting ongoing care is important. Ongoing care may include: Continuing therapeutic phlebotomy, Taking medicines as prescribed, Contacting your doctor right away about new or worsening symptoms or possible reactions to phlebotomy, Following up regularly with your doctor about test results, ongoing treatments, and annual exams, Using a diary or log to track the amount of iron in your body. […] If you need routine phlebotomy, you may have to change your usual work times to schedule your treatments. You also may have to change your work schedule to allow for periods of fatigue (tiredness) or recovery, especially if your treatment weakens you.

• #3 Patient education: Hereditary hemochromatosis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/hereditary-hemochromatosis-beyond-the-basics/print

  Phlebotomy can help prevent the potentially life-threatening complications of cirrhosis and liver cancer. […] Phlebotomy can resolve or markedly improve weakness, fatigue, lethargy, and darkening of the skin. […] Phlebotomy may resolve joint pain and heart disease. […] Chelation therapy refers to treatment with a drug that removes iron from the body. […] Although phlebotomy can alleviate or even completely resolve some complications of hemochromatosis, other measures may be necessary to treat complications that persist.

• #3 HFE hemochromatosis: an overview about therapeutic recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8885398/

  A phlebotomy schedule on the order of 400 – 500mL, considering body weight, weekly or every two weeks has been proposed. […] One phlebotomy every 1 to 4 months, depending on the patient’s iron status. […] Patients who have had iron overload should never stop checking his or her iron parameters and should be followed lifelong on an out-patient basis. […] Iron chelation therapy is not indicated for classical hemochromatosis. […] However, in rare cases, iron chelators are an adjuvant treatment (or alternative), such as in severe iron overload without efficacy with phlebotomies, poor vein conditions and severe non-HFE hemochromatosis (mainly juvenile form). […] Two current studies showed the importance of the adequate hemochromatosis treatment and of the early intervention to prevent morbidity caused by the hemochromatosis related to the HFE p.Cys282Tyr homozygosity.

• #3 Iron overload – Wikipedia

  https://en.wikipedia.org/wiki/Iron_overload

  Medications are used for those unable to tolerate routine blood draws, there are chelating agents available for use. The drug deferoxamine binds with iron in the bloodstream and enhances its elimination in urine and faeces. Typical treatment for chronic iron overload requires subcutaneous injection over a period of 8-12 hours daily. Two newer iron-chelating drugs that are licensed for use in patients receiving regular blood transfusions to treat thalassaemia (and, thus, who develop iron overload as a result) are deferasirox and deferiprone. […] A minimally invasive approach to hereditary hemochromatosis treatment is the maintenance therapy with polymeric chelators. These polymers or particles have a negligible or null systemic biological availability and they are designed to form stable complexes with Fe2+ and Fe3+ in the GIT and thus limiting their uptake and long-term accumulation. Although this method has only a limited efficacy, unlike small-molecular chelators, the approach has virtually no side effects in sub-chronic studies. Interestingly, the simultaneous chelation of Fe2+ and Fe3+ increases the treatment efficacy.

• #3

  https://www.nhs.uk/conditions/haemochromatosis/

  There’s currently no cure for haemochromatosis, but there are treatments that can reduce the amount of iron in the body and reduce the risk of damage. […] There are 2 main treatments. […] venesection (phlebotomy) a procedure to remove some of your blood; this may need to be done every week at first and can continue to be needed 2 to 4 times a year for the rest of your life […] chelation therapy where you take medicine to reduce the amount of iron in your body; this is only used if it’s not easy to regularly remove some of your blood. […] You do not need to make any big changes to your diet to control your iron levels if you’re having treatment, but you’ll usually be advised to avoid: breakfast cereals containing added iron, iron or vitamin C supplements, drinking too much alcohol.

• #4 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemochromatosis-Treatments.aspx

  Patients with hemochromatosis can improve their condition by measures such as avoiding all foods and supplements fortified with iron, avoiding vitamin C supplements, avoiding alcohol which predisposes to hepatic cirrhosis on its own, and avoiding seafood, especially shellfish and raw fish, as they may harbor some bacteria to which patients with hemochromatosis are more vulnerable. […] Once the iron levels decrease organ function improves. Patients experience less tiredness, relief in abdominal pain, and improvement of skin pigmentation. However, cirrhosis of the liver is irreversible and regular monitoring must be instituted to detect liver cancer early.

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